Case Report
Copyright ©The Author(s) 2019. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Jan 6, 2019; 7(1): 102-108
Published online Jan 6, 2019. doi: 10.12998/wjcc.v7.i1.102
Co-occurrence of IPMN and malignant IPNB complicated by a pancreatobiliary fistula: A case report and review of the literature
Xu Ren, Chun-Lan Zhu, Xu-Fu Qin, Hong Jiang, Tian Xia, Yong-Ping Qu
Xu Ren, Tian Xia, Digestive Disease Hospital of Heilongjiang Provincial Hospital, Harbin 150001, Heilongjiang Province, China
Chun-Lan Zhu, Xu-Fu Qin, The Second Digestive Department, Digestive Disease Hospital of Heilongjiang Provincial Hospital, Harbin 150001, Heilongjiang Provice, China
Hong Jiang, Department of Pathology of Heilongjiang Provincial Hospital, Harbin 150001, Heilongjiang Provice, China
Yong-Ping Qu, Digestive Endoscopy Center, Digestive Disease Hospital of Heilongjiang Provincial Hospital, Harbin 150001, Heilongjiang Provice, China
Author contributions: Ren X substantially contributed to the conception and design of the study and the acquisition, analysis, and interpretation of the data; Zhu CL, Qin XF, Jiang H, Xia T, and Qu YP contributed to drafting the article or making critical revisions related to important intellectual content of the manuscript; All authors approved the final version of the article to be published.
Informed consent statement: The patient provided written informed consent.
Conflict-of-interest statement: All authors declare no conflicts of interest.
CARE Checklist (2016) statement: All authors read, prepared, and revised the manuscript based on the CARE Checklist (2016).
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
Corresponding author: Xu Ren, MD, Chief Physician, Digestive Disease Hospital of Heilongjiang Provincial Hospital, Harbin150001, Heilongjiang Province, China.
Telephone: +86-451-88025065 Fax: +86-451-53625671
Received: September 27, 2018
Peer-review started: September 27, 2018
First decision: November 2, 2018
Revised: December 6, 2018
Accepted: December 7, 2018
Article in press: December 8, 2018
Published online: January 6, 2019

Intraductal papillary neoplasm of the bile duct (IPNB) is pathologically similar to intraductal papillary mucinous neoplasm (IPMN). However, there are several significant differences between them. The rate of IPMN associated with extrapancreatic malignancies has been reported to range from 10%-40%, and it may occasionally be complicated with the presence of fistulas. IPMN associated with malignant IPNB is extremely rare and only nine cases have been reported in the literature.


We report a 52-year-old man who presented with recurrent cholangitis for nine months. Computed tomography and magnetic resonance cholangiopancreatography showed the common bile duct stricture with dilated pancreatobiliary duct without other abnormal findings. The underlying pathogenesis could not be identified based on the radiologic images. Endoscopic retrograde cholangiopancreatography revealed a pancreatobiliary fistula with dilated main pancreatic duct, biliary stricture with dilated biliary tree, and mucus discharge from the enlarged orifice of the major papilla. The patient underwent SpyGlass cholangiopancreatoscopy due to a suspected mucin-producing biliary neoplasm and indeterminate main pancreatic duct dilatation. Multiple papillary growing neoplasms with vascular images, with the extent of lesions spreading in the biliopancreatic ductal lumens, were identified by SpyGlass. In addition, the presence of a pancreatobiliary fistula was also identified. The patient was diagnosed as having benign IPMN and malignant IPNB with focal invasion by postoperative pathology. Furthermore, varying histological subtypes were present in both IPMN and IPNB. Pylorus-preserving pancreaticoduodenectomy was performed on the patient with excellent results during the 52 month follow-up period.


We deemed that pancreatography and SpyGlass allowed for an efficient diagnosis of IPMN with pancreatobiliary fistula, whereas the etiology could not be identified by radiologic imaging.

Keywords: Intraductal papillary mucinous neoplasm of the pancreas, Intraductal papillary neoplasm of the bile duct, Extrapancreatic malignancies, Co-occurrence, Pancreatobiliary fistula, SpyGlass cholangiopancreatoscopy, Endoscopic retrograde cholangiopancreatography, Case report

Core tip: We report a patient with an extremely rare co-occurrence of intraductal papillary mucinous neoplasm (IPMN) and malignant intraductal papillary neoplasm of the bile duct (IPNB) accompanied with a pancreatobiliary fistula. The etiology of biliopancreatic duct dilatations could not be identified by radiologic imaging. After the patient underwent endoscopic retrograde cholangiopancreatography, the presence of a pancreatobiliary fistula was revealed by pancreatography. SpyGlass cholangiopancreatoscopy showed multiple papillary neoplasms in the pancreaticobiliary duct. In addition, SpyGlass was able to identify a pancreatobiliary fistula. The patient was diagnosed with benign IPMN and malignant IPNB. Different histological subtypes for both IPMN and IPNB were also identified. Radical resection on the patient achieved excellent results.